No cure for Huntington’s disease has been found but there are therapeutic solutions for palliating some of its symptoms.(Frank, S. 2009)

Surgical interventions are not usually performed. 

 

Drugs dosage depends on the patient. So personal treatment is performed and it is based on the daily life of the patient and expert opinion.

 

Dopaminergic drugs are not prescribed clinically. Antiparkinsonian drugs have been administered to treat hypokinesia but they have not represented an improvement. Atypical and typical neuroleptics and depleting agents are used for treating chorea and hyperkinesia. Neuroleptics are dopamine receptor blocking.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Should the patient loss be unable to live on his own, help may be found in day-care institutions. Huntington’s disease organizations can give support to patients and caregivers.

 

No pharmacological and therapeutic interventions such as neuroprotective and disease-delaying drugs have been reported to heal or delay HD onset yet.

 

 

Drugs

 

• Clozapine: it is an atypical antipsychotic drug. Its side effects make it less practical because weekly monitoring of the patients is needed. Agranulocytosis and seizures are some of its side effects so white cell monitoring is needed

 

• Tetrabenzine (TBZ): it is a dopamine-depleting drug. The study of the Huntington study group (HSG) demonstrated TBZ suppresses chorea for up 80 weeks (Frank, 2009) 

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 Antichoreatic drugs

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Anti-depression and anti- aggression drugs

 

These are the main neuropsychiatric symptomatology in Huntington’s disease. Both signs are devastating to the relatives of the patient.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Experts specialised in different fields get involved in Huntington’s disease treatment. So, a multidisciplinary team performs treatment. Non-medication interventions are recommended and some of them are speech and occupational therapy, psychologist and dietician counselling and physiotherapy. Treatment must be individualised and its goal is improving quality of life.

 

 

 

Therapies under investigation

 

 

Fetal striatal tissue transplantation

 

University of South Florida made a clinical trial of human fetal striatal tissue transplantation. There was no immunity response against transfected tissue and no huntingtin-altered aggregations were found in transplant region. Innervation by host tyrosine hydroxylase fibres was found in these regions. (Friedlander, RM. 2003)

 

 

 

Aggregation reducing

 

GLUT 1 (a glucose transporter involved in glucose metabolism) was involved in polyglutamine toxicity. GLUT1 overexpression reduced this toxicity by decreasing huntingtin aggregation. Raised glucose concentration (8g/L) in culture medium and 2DOG (2-deoxyglucose) produce the same effect. This protective effect was associated with autophagy’s increase and decrease of phosphorylation of mTOR (Mamalian target of rapamycin, regulator of autophagy). (Ravikumar et al, 2003)

 

 

 

MiR-196a overexpression

 

 

MicroRNAs (miRNAs) are involved in genes deregulation in Huntington’s disease. MiR-196a (miRNA) overexpression in HD transgenic mice reduced mutated huntingtin in brain and neuropathological progression got improved. This miRNA reduced Huntingtin expression and aggregation when induced stem cells were differentiated into neurons. MiR-196a is involved in alteration of gliosis, systems of ubiquitin-proteasome, neuronal pathways… It can become a therapeutic target. (Cheng et al, 2013)